Biomedical subjects
Publications and source records attributed to M Aladjem.
A 9-year-old boy presented with recurrent episodes of renal colic. One year later Wilson disease was diagnosed. Evaluation of liver function and assessment of serum copper, caeruloplasmin concentration and urinary copper excretion in any child presenting with nephrolithiasis is suggested.
Serum creatine kinase (CK) was determined in 52 children admitted following an episode of febrile convulsions. Enzyme levels correlated with the estimated duration of the seizure. Twenty-four hour values were significantly higher than those observed 1 hour after the convulsive episode. Serum CK levels are frequently used for diagnostic purposes, so the questionable validity of this test when drawn after a convulsive episode must be considered.
Several investigators have reported recently that in rats, oral calcium load is associated with a marked amelioration in gentamicin-induced renal failure. In contrast to these reports, using the same animal model, we could not observe calcium-induced moderation in gentamicin nephrotoxicity as reflected by either urea or creatinine serum concentration or by various renal cortical intracellular enzymatic activities. Similarly, verapamil, a calcium channel blocker, had no effect on the degree of renal failure in these animals. We conclude that manipulation of calcium diet may not be uniformly effective in reducing gentamicin nephrotoxicity. Additional nutritional factors may play a crucial role in achieving the amelioration of this model of toxic nephropathy.
Nineteen children with the minimal change form of nephrotic syndrome were divided according to their pattern of response to prednisone: steroid-dependent and frequent relapsers. All patients received cyclophosphamide for 56 days in a single daily dose of 2.5 mg/kg (total 140 mg/kg), in order to prolong the length of remission. The percentage of patients who continued in remission at the end of the 1st, 2nd and 5th years was greater in the frequent-relapser group. This retrospective analysis confirms that the pattern of response to prednisone may be an important criterion for the selection of patients who will benefit from cyclophosphamide therapy.
A 4 1/2-year-old boy with macrocephaly, pseudopapilledema, lipoangiomatosis, macropenia, and spotted pigmentations of the glans is reported. Lipoid masses were found in the subcutaneous tissue, tonsils, and probably the left lung. Some of these findings are consistent with features already reported by Riley and Smith, later by Bannayan, and recently by Ruvalcaba et al. We propose to unify the features of this syndrome and name it macrocephaly, hamartomas, and papilledema syndrome. The inheritance in our described case seems to be autosomal dominant.
A rapid and excessive enlargement in occipito frontal circumference (OFC) as the only manifestation of multiple cerebral abscesses in infancy is extremely rare. A 2-month-old asymptomatic infant presenting with increasing OFC is described. Surgical drainage of the cerebral abscesses followed by prolonged antibiotic therapy resulted in complete recovery.
Four newborn babies developed acute heart failure a few hours after circumcision at 8 days. During this procedure, a sponge soaked in epinephrine was applied to the circumcision site and left there for several hours. Treatment was with digoxin and diuretics and signs of heart failure disappeared within 24-72 hours.
One hundred and thirty four ambulatory children with bronchial asthma were investigated in the Pediatric Pulmonary-Allergic Service. In 95 patients an interval characterised by prodromal respiratory symptoms (cough, rhinorrhoea, and wheezing), behavioural changes (irritability, apathy, anxiety, and sleep disorders), gastrointestinal symptoms (abdominal pain and anorexia), fever, itching, skin eruptions, and toothache preceded the onset of the attack of asthma. Each child had his own constant set of prodromal findings. A significant age related increase in serum IgE concentrations was observed in these patients. No such relation was observed in children with an acute onset of attack of asthma without any preceding symptoms. We suggest that awareness of these prodromal symptoms may lead to an early introduction of treatment, thus avoiding or abbreviating some of the acute attacks of asthma.
The effects of unilateral nephrectomy and the impact of gentamicin administration on renal tissue enzyme activities in adult Wistar rats were investigated. Gentamicin 200 mg/kg body wt. or an equivalent volume of saline to control rats was administered subcutaneously on three consecutive days, followed by unilateral nephrectomy. Rats were killed on day 3, 7 or 14 following nephrectomy. Alkaline phosphatase, predominantly a proximal tubular brush border enzyme, rose in both the experimental and control groups, however, significantly less in the gentamicin treated rats. Aspartate aminotransferase activity, an enzyme participating in renal glucogenesis, increased transiently in the control but remained unchanged in the experimental group. No difference in glucose-6-phosphate dehydrogenase activity between the two groups was observed, probably reflecting the localization of this enzyme to distal tubular segments, a site unaffected by gentamicin. Significant and similar increases in Mg2+ and Na+ K+ ATPase were observed on day 14 in both groups. The administration of the drug resulted in a marked reduction in oxygen consumption, with a higher oxidation to phosphorylation ratio (P/O). Serum creatinine concentration was significantly higher on days 3 and 7 in the experimental group reverting to control values on the 14th day. Urea concentration increased significantly on days 3 and 7, decreasing on the 14th day to values slightly, but significantly, higher than those of the controls.
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A 5-month-old infant presented with severe dyspnea and dysphagia resulting from a right-sided cervical mass. At 5 months of age, a large aberrant thymus was excised, resulting in the disappearance of all symptoms. Pathological examination showed normal thymus tissue. Since the preoperative chest X-ray film showed a normal thymic shadow and the T-lymphocyte functions were normal, we conclude that this was not an ectopic gland but an undescended thymic implant.
The effect of digitalis and diuretic therapy on sweat fluid composition was investigated. Patients treated for congestive heart failure with a combination of digoxin and diuretics demonstrated a higher concentration of sodium and chloride in their sweat fluid when compared to age-matched controls. The administration of diuretics alone did not affect sweat fluid composition. The digoxin-induced increase in sodium concentration was significantly higher than that observed for chloride. These data suggest that digoxin markedly inhibits sodium reabsorption along the sweat gland tubule, whereas chloride transport is affected to a lesser degree. A significant correlation between sweat fluid sodium and serum digoxin concentrations was observed. We conclude that the increased digoxin-induced sweat sodium and chloride losses may affect sodium homeostasis in patients with congestive heart failure.
A two-year-old boy of Arabic extraction presented with progressive jaundice and prolonged pyrexia. Both IgM and IgG immunofluorescent antibody titers for Q fever were 1:1280. Two goats and one cow of the domestic animals owned by the family also had positive antibody titers against Q fever. In spite of antibiotic treatment with tetracyclines and chloramphenicol, the hepatic involvement progressed gradually. On the twentieth day of admission the child succumbed from hepatic failure. This child presents a rare case of fatal hepatic failure due to Q fever.
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The urinary calcium/creatinine ratio, during a low-calcium diet and after an oral calcium load, was determined in 128 members of 29 families. No differences were observed between subjects of different sexes, ages and ethnic origins. Our data allow characterization of hypercalciuria in Israeli patients with urolithiasis.
Renal effects of the administration of contrast media during cardiac catheterisation were compared in two groups of patients with congenital heart diseases. Group A consisted of 21 patients with cardiac malformations, characterised primarily by left ventricular volume overload and known to be associated occasionally with left heart failure, such as: left to right shunts and left ventricular valvular defects. Group B consisted of 23 patients with lesions affecting the right ventricle which are rarely associated with left heart failure, such as: Tetralogy of Fallot and Pulmonic stenosis. Patients in Group A showed a significant increment in both plasma creatinine and uric acid levels in the 24 h following heart catheterisation. This observation was significantly more prominent in the older age group (above the age of 5 years). In Group B no changes in these parameters were encountered. Plasma renin activity and fractional sodium excretion increased and decreased respectively, by a similar degree in both groups in the 24 h following contrast media administration. No difference in renal tubular handling of uric acid was observed between both groups, nor did any of the patients studied demonstrate any degree of proteinuria or abnormality in the urine sediment, prior to or following heart catheterisation. We suggest that chronic pre-existing left ventricular overload should be considered a risk factor among the other known risk factors which promote the incidence of acute renal failure after contrast media administration.(ABSTRACT TRUNCATED AT 250 WORDS)
The mean serum aldosterone concentration of 37 infants with acute gastroenteritis and dehydration was markedly elevated on admission (mean +/- SE 94.3 +/- 12.1 ng/ml) and approximated to normal values (18.2 +/- 3.7 ng/ml) following recovery from the acute disease (t=3.56 p less than 0.005). Serum aldosterone levels were significantly positively correlated with the percent weight loss (r=0.41, p less than 0.05) and with the blood urea nitrogen levels (r=0.55, p less than 0.001). There was no correlation between either serum sodium levels or blood osmolarity and aldosterone concentrations. Serum potassium levels were positively correlated with aldosterone levels (r=0.53, p less than 0.001). These findings indicate that small infants when dehydrated respond appropriately with elevated aldosterone levels. The amount of body fluid depletion and hyperkalemia are the major factors determining the amount of aldosterone response.